TDP-43 pathologic lesions and clinical phenotype in frontotemporal lobar degeneration with ubiquitin-positive inclusions.

TDP-43 pathologic lesions and clinical phenotype in frontotemporal lobar degeneration with ubiquitin-positive inclusions. Research Abstract Details 

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TDP-43 pathologic lesions and clinical phenotype in frontotemporal lobar degeneration with ubiquitin-positive inclusions. Abstract Text:

Murray Grossman,Elisabeth M Wood,Peachie Moore,Manuela Neumann,Linda Kwong,Mark S Forman,Christopher M Clark,Leo F McCluskey,Bruce L Miller,Virginia M-Y Lee,John Q Trojanowski,Murray Grossman,Elisabeth M Wood,Peachie Moore,Manuela Neumann,Linda Kwong,Mark S Forman,Christopher M Clark,Leo F McCluskey,Bruce L Miller,Virginia M-Y Lee,John Q Trojanowski,

BACKGROUND: TDP-43 is a major ubiquitinated disease protein in the pathologic condition of frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U). OBJECTIVE: To investigate the demographic, clinical, and neuropsychological features associated with subtypes of FTLD-U with TDP-43 inclusions (FTLD-U/TDP-43). DESIGN: Retrospective clinical-pathologic study. SETTING: Academic medical center. Patients Twenty-three patients with histopathologically proven FTLD-U. MAIN OUTCOME MEASURES: Demographic, symptom, neuropsychological, and autopsy characteristics. RESULTS: There are notably different clinical and neuropsychological patterns of impairment in FTLD-U subtypes. Patients with FTLD-U/TDP-43 characterized by numerous neuronal intracytoplasmic inclusions have shorter survival; patients with FTLD-U/TDP-43 featuring numerous neurites have difficulty with object naming; and patients with FTLD-U/TDP-43 in whom neuronal intranuclear inclusions are present have substantial executive deficits. There are also different anatomical distributions of ubiquitin pathologic features in FTLD-U subgroups, consistent with their cognitive deficits. CONCLUSION: Distinct TDP-43 profiles may affect clinical phenotypes differentially in patients with FTLD-U.

TDP-43 pathologic lesions and clinical phenotype in frontotemporal lobar degeneration with ubiquitin-positive inclusions. Publishing Authors By Initials

M Grossman,EM Wood,P Moore,M Neumann,L Kwong,MS Forman,CM Clark,LF McCluskey,BL Miller,VM Lee,JQ Trojanowski,M Grossman,EM Wood,P Moore,M Neumann,L Kwong,MS Forman,CM Clark,LF McCluskey,BL Miller,VM Lee,JQ Trojanowski,

For similar proteins: ubiquitins: ubiquitin research abstracts see: proteins: ubiquitins: ubiquitin research

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TDP-43 pathologic lesions and clinical phenotype in frontotemporal lobar degeneration with ubiquitin-positive inclusions. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: Archives of neurology

VOLUME: 64

Page Numbers: 1449-54

Journal Abbreviation: Arch. Neurol.

ISSN: 0003-9942

DAY: 20

MONTH: Oct

YEAR: 2007

TDP-43 pathologic lesions and clinical phenotype in frontotemporal lobar degeneration with ubiquitin-positive inclusions. Information

Number of References:

LANGUAGE: eng

NlmUniqueID: 372436

TDP-43 pathologic lesions and clinical phenotype in frontotemporal lobar degeneration with ubiquitin-positive inclusions. Keywords Mesh Terms:

KEYWORDS: Ubiquitin

MESH TERMS: metabolism

Chemical & Substance for Abstract: TDP-43 pathologic lesions and clinical phenotype in frontotemporal lobar degeneration with ubiquitin-positive inclusions. Information

Substance Name: protein TDP-43

Registry Number: 0

Grant and Affiliation Information for TDP-43 pathologic lesions and clinical phenotype in frontotemporal lobar degeneration with ubiquitin-positive inclusions.

AFFILIATION: Department of Neurology, University of Pennsylvania School of Medicine, 2 Gibson, 3400 Spruce St, Philadelphia, PA 19104-4283, USA. mgrossma@mail.med.upenn.edu

Country: United States

AGENCY: United States NINDS

GRANT: NS44266

ACRONYM: NS

MEDLINETA: Arch Neurol

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